Title |
Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options
|
---|---|
Published in |
Journal of Blood Medicine, July 2015
|
DOI | 10.2147/jbm.s71319 |
Pubmed ID | |
Authors |
Tia Solh, Ashley Botsford, Melhem Solh |
Abstract |
Glanzmann's thrombasthenia (GT) is a genetic platelet surface receptor disorder of GPIIb/IIIa (ITG αIIbβ3), either qualitative or quantitative, which results in faulty platelet aggregation and diminished clot retraction. Spontaneous mucocutaneous bleeding is common and can lead to fatal bleeding episodes. Control and prevention of bleeding among patients with GT is imperative, and remains challenging. Local measures, including anti-fibrinolytic therapy, with or without platelet transfusions, used to be the mainstay of therapy. However, in recent years the use of recombinant factor VIIa (rFVIIa) has increased significantly, with excellent response rates in treating and preventing hemorrhage among GT patients. Gene therapy and stem cell transplantation offer a potential cure of this disease, but both are costly and remain experimental at this point. This manuscript offers a comprehensive review of our understanding of GT and the available treatment options. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 1 | 33% |
Unknown | 2 | 67% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 2 | 67% |
Practitioners (doctors, other healthcare professionals) | 1 | 33% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Netherlands | 1 | <1% |
Unknown | 104 | 99% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Bachelor | 18 | 17% |
Student > Ph. D. Student | 10 | 10% |
Student > Master | 7 | 7% |
Student > Postgraduate | 6 | 6% |
Student > Doctoral Student | 5 | 5% |
Other | 19 | 18% |
Unknown | 40 | 38% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 36 | 34% |
Biochemistry, Genetics and Molecular Biology | 11 | 10% |
Agricultural and Biological Sciences | 5 | 5% |
Immunology and Microbiology | 3 | 3% |
Chemistry | 2 | 2% |
Other | 5 | 5% |
Unknown | 43 | 41% |